초록 열기/닫기 버튼
Adult onset Still’s disease (AOSD) is a systemic autoinflammatory disorder that presents with recurrent fever, extreme fatigue,and joint pain. Pulmonary involvement is not uncommon and, although rare, severe pneumonitis can progress to respiratory failure. Still’s disease-associated pneumonitis is generally treated with immunosuppressive agents, but improvement in our understandingof systemic inflammatory processes led us to explore alternative agents. Anakinra is an interleukin-1 receptor antagonist used totreat autoinflammatory disorders resistant to immunosuppressive therapy. Several case reports have demonstrated efficacy ofanakinra in treating AOSD, but its relevance in cases complicated with severe pneumonitis has not been examined. Our patient’sdisease activity was not controlled with systemic steroids and cyclophosphamide. Treatment with anakinra led to a dramatic clinicalresponse. This is the first reported case of AOSD with severe pneumonitis refractory to conventional therapy successfully treatedwith anakinra.
키워드열기/닫기 버튼
Adult onset Still’s disease, Pneumonitis, Anakin
